Thirty-Year Survival After Cardiac Surgery in Children With Williams-Beuren Syndrome (from the Pediatric Cardiac Care Consortium Study)

Williams-Beuren syndrome (WBS) is a genetic condition frequently requiring interven-tions for associated congenital heart disease (CHD). Long-term survival data after cardiac interventions for children with WBS are sparse. This is a retrospective cohort study aim-ing to describe the 30-year survival outcomes of children with WBS after interventions for CHD using the Pediatric Cardiac Care Consortium (PCCC), a large North American -based registry of interventions for pediatric heart diseases, between 1982 and 2009. Out-comes were obtained from the PCCC and by linkage with the National Death Index through 2020. Survival of patients with WBS and their major subgroups was assessed by Kaplan-Meier survival curves and Cox regression. A total of 200 patients met the inclusion criteria of having their first intervention for CHD at a US PCCC center and age < 21 years at time of intervention. The most common lesions were left heart obstructive lesions (LHOL), either in isolation (37%) or in combination with right heart obstructive lesions (RHOL) (49.0%), whereas isolated RHOL accounted for 11% of the total. The first proce-dure was surgery for 85.5% of the group, and the remainder underwent a transcatheter procedure. There were 5 in-hospital deaths (2.5%), and among survivors to hospital dis-charge, 164 had sufficient identifiers for National Death Index linkage. Over a median period of postdischarge follow-up of 23.7 years (interquartile range 18.7 to 27.3), 16 deaths occurred, with an overall 30-year survival rate of 90%. Survival rates ranged from 96.1% for isolated LHOL or RHOL to 83.4% for patients with combined disease (adjusted haz-ard ratio 4.7, 95% confidence intervals 1.35 to 16.59). (c) 2022 Elsevier Inc. All rights reserved. (Am J Cardiol 2023;187:48-53)

Automated summary

This is a retrospective cohort study examining the 30-year survival outcomes of children with Williams-Beuren syndrome (WBS) who underwent interventions for congenital heart disease (CHD). The study found that the overall 30-year survival rate was 90%, with varying survival rates among different subgroups.